Comparison of echocardiographic parameters in Fabry cardiomyopathy and light-chain cardiac amyloidosis
Language English Country United States Media print-electronic
Document type Comparative Study, Journal Article
PubMed
30247786
DOI
10.1111/echo.14144
Knihovny.cz E-resources
- Keywords
- Fabry disease, diastolic function, echocardiography, restrictive cardiomyopathy, systolic function,
- MeSH
- Amyloidosis diagnostic imaging physiopathology MeSH
- Ventricular Dysfunction, Left diagnostic imaging physiopathology MeSH
- Echocardiography methods MeSH
- Fabry Disease diagnostic imaging physiopathology MeSH
- Cardiomyopathies diagnostic imaging physiopathology MeSH
- Middle Aged MeSH
- Humans MeSH
- Heart Diseases diagnostic imaging physiopathology MeSH
- Ventricular Remodeling physiology MeSH
- Reproducibility of Results MeSH
- Retrospective Studies MeSH
- Aged MeSH
- Sensitivity and Specificity MeSH
- Heart Ventricles diagnostic imaging physiopathology MeSH
- Check Tag
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Comparative Study MeSH
BACKGROUND: Fabry cardiomyopathy (FC) and light-chain amyloid cardiomyopathy (AL) present with concentric left ventricular (LV) hypertrophy/remodeling and diastolic rather than systolic dysfunction. Direct comparisons are difficult due to rarity and confounded by variability of LV thickness. AIMS: To compare LV diastolic and systolic properties between patients with FC and AL in a cohort matched for interventricular septal thickness (IVS). METHODS: A two-center echocardiographic analysis was performed, comprising 118 patients with IVS ≥12 mm (FC and AL 59 patients each) matched by IVS. RESULTS: Fabry cardiomyopathy patients had larger LV end-diastolic diameter (47.7 [44.0-50.9] vs 45.0 [41.5-49.0] mm, P = 0.002), better LV ejection fraction (EF 68.7 [63.4-74.0] vs 63.0 [54.0-70.0]%, P = 0.001) and midwall fractional shortening (midFS 14.8 [13.0-16.1] vs 12.1 [8.9-15.0]%, P = 0.006). LV EF <40% was rare in both (2% vs 7%, P = 0.17). AL patients expressed higher LV diastolic dysfunction grade (III in 26% vs 4%, II in 21% vs 12% and I in 54% vs 84%, P = 0.004), with higher E/e' ratio (13.6 [10.2-18.8] vs 9.8 [7.5-12.3], P < 0.0001). Average E/e' ratio and midFS were significantly associated with NYHA severity in both groups (P < 0.05 for all). CONCLUSIONS: Matched AL patients had worse LV diastolic function than FC, driven by E/e'. Significant LV systolic dysfunction was rare overall. MidFS and E/e' were associated with heart failure severity in both groups.
References provided by Crossref.org
Narrative review on Morbus Fabry: diagnosis and management of cardiac manifestations
